Clinical Review
Acquired ichthyosis

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Acquired ichthyosis (AI) is a nonhereditary cutaneous disorder characterized by dry, rough skin with prominent scaling that involves significant portions of the body. It has been associated with malignancies; autoimmune/inflammatory, metabolic, endocrine, and infectious diseases; and medication use. Most microscopic studies of AI exhibit hyperkeratosis with a reduced or absent granular layer. Because AI has been linked to a variety of conditions, the workup of a patient presenting with this finding can be complex. We present an update on AI to provide clinicians with direction regarding the assessment and treatment of patients presenting with AI. An algorithm for the evaluation of patients presenting with AI is provided.

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Clinical findings

Acquired ichthyosis (AI) most commonly manifests in adulthood and is otherwise difficult to distinguish from the hereditary forms of ichthyosis.3 Clinical manifestations include symmetric scaling, which ranges in severity from minor roughness and dryness to dramatic desquamation of platelike scales (Fig 1).4 The color of the scales varies from white to gray to brown, with a diameter ranging from less than 1 mm to greater than 1 cm.3 It primarily affects the trunk and limbs, typically being

Pathology

Histologically, both AI and ichthyosis vulgaris (IV) usually present with compact or laminated orthohyperkeratosis, a reduced or absent granular layer, and normal thickness of the spinous layer.1, 6 Absence of an inflammatory infiltrate in the dermis is also among the typical histologic findings.7, 8 Alternatively, epidermal atrophy and presence of a mild perivascular lymphohistiocytic infiltrate in the papillary dermis have also been observed.6

Pathogenesis

Ichthyosis occurs when there is a disruption in the process of cornification, resulting in hyperkeratosis, scaling, and abnormalities of the stratum corneum barrier function. A thickened stratum corneum is the result of cells entering this compartment at an increased rate or prolonged retention of corneocytes.

There are numerous ichthyosiform dermatoses, and these disorders all have in common abnormal cornification. IV, which is the most common inherited ichthyosis, is caused by mutations in

Diagnosis

The first step in evaluating an adolescent or adult with new-onset ichthyosis is differentiating AI from other causes of new-onset ichthyosis, such as late-onset IV, xerosis, and Refsum's disease. Essentially all other hereditary forms of ichthyosis will present well before age 13 years and, therefore, do not need to be considered in this setting.

IV is an autosomal dominant disease and, therefore, a positive family history is typical, unlike in AI. Patients develop dry, rough skin that usually

Association with systemic diseases

Once the diagnosis of AI is established, focus must change to evaluating the patient for underlying causes. AI may be a result of malignant disease, nonmalignant disease, or a drug reaction (Table I). If associated with a systemic disease, the cutaneous manifestations can present either before or after identification of the associated disease.5 The severity of AI may depend on the severity and acuteness of the internal disease.11 The ichthyosis typically regresses once the underlying disease

Medications

Cimetidine, a commonly used histamine blocker,69 has been reported to cause AI.51, 69, 70 It has been shown that in addition to its histamine-blocking effects, cimetidine is also a competitive inhibitor of dihydrotestosterone, and ichthyosis may be related to this antiandrogenic activity.6

Clofazimine, a riminophenazine derivative that is used in the management of leprosy,6, 71 has been linked to AI.6, 51, 71 Ichthyosis arises only when the dosage reaches 100 mg/d.71 In a study of 8 patients

Diagnostic considerations

Once the diagnosis of AI is established or strongly suggested, the initial focus must be identifying an underlying cause. It is important to remember that the skin manifestations may appear before or after the identification of underlying disease. The most important initial step in the evaluation is a detailed history, review of symptoms, and physical examination.

Specific questioning should inquire about medication use, risk factors for HIV, constitutional symptoms, history of liver or kidney

Treatment

For general treatment, hydration, lubrication, and keratolysis are all useful.10 In addition, the impaired barrier function of the skin associated with ichthyosis predisposes patients to skin infections.10 Because of this predisposition, consideration should be given to prophylactic measures, such antiseptic soaps containing triclosan or chlorhexidine. If skin infections develop, topical and systemic antibacterials should be quickly instituted.10

Hydration encourages desquamation by increasing

Conclusion

The management of AI is challenging and complex because of its association with numerous systemic diseases and drugs. We have reviewed the recent literature on this topic and have offered suggestions for the evaluation of these patients.

Because there are a large number of diseases, medications, and physiologic conditions reported in association with AI, the diagnostic evaluation must be conducted in a thorough, organized manner. In general, treatment should be directed at the underlying disease

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